Gorham–Stout syndrome affecting the temporal bone with cerebrospinal fluid leakage

Morimoto, Noriko; Ogiwara, Hideki; Miyazaki, Osamu; Kitamuara, Masayuki; Nishina, Sachiko; Nakazawa, Atsuko; Maekawa, Takanobu; Morota, Nobuhito

doi:10.1016/j.ijporl.2013.06.004

Cited by 32 publications

(25 citation statements)

References 7 publications

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“…The pathogenesis of GSD is poorly understood. There are only three reported cases of hearing loss secondary to GSD . In one of these patients, hearing loss was caused by an osteolytic lesion directly infiltrating the cochlear duct.…”

Section: Discussionmentioning

confidence: 99%

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Gorham-Stout Disease of the Skull Base With Hearing Loss: Dramatic Recovery and Antiangiogenic Therapy

Nozawa

Ozeki

Kuze

et al. 2015

Pediatr Blood Cancer

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Gorham-Stout disease (GSD) is a rare disorder of unknown etiology. We present a 6-year-old male with GSD involving the skull base who presented with recurrent cerebrospinal fluid (CSF) rhinorrhea, severe hearing loss, and facial palsy secondary to cerebellar herniation into the internal auditory canal. After 2 months of treatment with pegylated interferon (IFN) α-2b (50 μg/week), his hearing recovered dramatically. Two years later, new bone formation appeared radiologically and IFN was switched to sirolimus. One year after the switch, CSF rhinorrhea disappeared. Antiangiogenic therapy might inhibit proliferation of vascular endothelial cells in osteolytic lesions and lead to new bone formation.

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Section: Discussionmentioning

confidence: 99%

“…There are only three reported cases of hearing loss secondary to GSD. [9][10][11] In one of these patients, hearing loss was caused by an osteolytic lesion directly infiltrating the cochlear duct. However, the causes of hearing loss in the other two patients were unclear.…”

Section: Discussionmentioning

confidence: 99%

Gorham-Stout Disease of the Skull Base With Hearing Loss: Dramatic Recovery and Antiangiogenic Therapy

Nozawa

Ozeki

Kuze

et al. 2015

Pediatr Blood Cancer

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“…С учетом вариабельности скорости остеолиза наиболее перспективными биомаркерами его активности счита-ются VGEF-A [7,10,37] и интерлей-кин-6 [12,17,18,43], уровень которых при GSD может быть повышен. Вме-сте с тем показатели этих цитокинов могут оставаться и нормальными [25,37], поэтому продолжаются активные поиски других биомаркеров остеоли-за -VEGF-C [37], PDGF-BB [25], sRANKL и остеопротегерина [18].…”

Section: рисunclassified

“…Вме-сте с тем показатели этих цитокинов могут оставаться и нормальными [25,37], поэтому продолжаются активные поиски других биомаркеров остеоли-за -VEGF-C [37], PDGF-BB [25], sRANKL и остеопротегерина [18].…”

Section: рисunclassified

A Rare Variant of Angiogenic Vertebral Body Destruction in a Child: Gorham’s Disease?

Мушкин¹,

Евсеев²,

Николаев³

et al. 2018

Hir. pozvonoč.

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“…To date, 4 cases of GSD involving CSF leakage have been published, 3,9,17,18 all presenting with lesions involving the petrous and temporal bones or the spinal vertebral bodies (Table 1). In this report we discuss the case of a patient with GSD who presented to our clinic with clinical signs of intracranial hypotension due to CSF leakage as a result of progression of a thoracic vertebral body lesion involving the dura mater with a consecutive CSF fistula.…”

mentioning

confidence: 99%

Cerebrospinal fluid leakage in Gorham-Stout disease due to dura mater involvement after progression of an osteolytic lesion in the thoracic spine

Molina¹,

Niederstadt²,

Ruland

et al. 2014

SPI

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Patients with Gorham-Stout disease (GSD), a rare disease of poorly understood etiopathophysiology, suffer from progressive osteolysis. Destruction of bone matrix is caused by lymphatic vessels, which can lead to CSF leakage if parts of bony structures adjacent to CSF spaces are involved. So far, fewer than 200 patients have been reported in the literature; only 4 of these patients presented with CSF leakage. The authors report the case of a 30-year-old man with GSD and CSF leakage due to dura mater involvement after progression of an osteolytic lesion in the thoracic spine. Neurosurgical intervention, including dura repair, was needed. Experimental medical therapy with rapamycin was started, leading to disease control for more than 12 months. Progression of GSD can lead to destruction of the meninges, causing CSF leakage. The authors review 4 other cases reported in the literature and discuss therapeutic options.

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Gorham–Stout syndrome affecting the temporal bone with cerebrospinal fluid leakage

Cited by 32 publications

References 7 publications

Gorham-Stout Disease of the Skull Base With Hearing Loss: Dramatic Recovery and Antiangiogenic Therapy

Gorham-Stout Disease of the Skull Base With Hearing Loss: Dramatic Recovery and Antiangiogenic Therapy

A Rare Variant of Angiogenic Vertebral Body Destruction in a Child: Gorham’s Disease?

Cerebrospinal fluid leakage in Gorham-Stout disease due to dura mater involvement after progression of an osteolytic lesion in the thoracic spine

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